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Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems

Overview of attention for article published in Acta Neuropathologica, February 2012
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  • Above-average Attention Score compared to outputs of the same age (55th percentile)

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116 Mendeley
Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems
Published in
Acta Neuropathologica, February 2012
DOI 10.1007/s00401-012-0954-z
Pubmed ID

Fausto J. Rodriguez, Andrew L. Folpe, Caterina Giannini, Arie Perry


Peripheral nerve sheath tumors are common neoplasms, with classic identifiable features, but on occasion, they are diagnostically challenging. Although well-defined subtypes of peripheral nerve sheath tumors were described early in the history of surgical pathology, controversies regarding the classification and grading of these tumors persist. Advances in molecular biology have provided new insights into the nature of the various peripheral nerve sheath tumors, and have begun to suggest novel targeted therapeutic approaches. In this review, we discuss current concepts and problematic areas in the pathology of peripheral nerve sheath tumors. Diagnostic criteria and differential diagnosis for the major categories of nerve sheath tumors are proposed, including neurofibroma, schwannoma, and perineurioma. Diagnostically challenging variants, including plexiform, cellular and melanotic schwannomas are highlighted. A subset of these affects the childhood population, and has historically been interpreted as malignant, although current evidence and outcome data suggest they represent benign entities. The growing current literature and the author's experience with difficult to classify borderline or "hybrid tumors" are discussed and illustrated. Some of these classification gray zones occur with frequency in the gastrointestinal tract, an anatomical compartment that must always be entertained when examining these neoplasms. Other growing recent areas of interest include the heterogeneous group of pseudoneoplastic lesions involving peripheral nerve composed of mature adipose tissue and/or skeletal muscle, such as the enigmatic neuromuscular choristoma. Malignant peripheral nerve sheath tumors (MPNST) represent a diagnostically controversial group; difficulties in grading and guidelines to separate "atypical neurofibroma" from MPNST are provided. There is an increasing literature of MPNST mimics which neuropathologists must be aware of, including synovial sarcoma and ossifying fibromyxoid tumor. Finally, we discuss entities that are lacking from the section on cranial and paraspinal nerves in the current WHO classification, and that may warrant inclusion in future classifications. In summary, although the diagnosis and classification of most conventional peripheral nerve sheath tumors are relatively straightforward for the experienced observer, yet borderline and difficult-to-classify neoplasms continue to be problematic. In the current review, we attempt to provide some useful guidelines for the surgical neuropathologist to help navigate these persistent, challenging problems.

Twitter Demographics

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Mendeley readers

The data shown below were compiled from readership statistics for 116 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Germany 2 2%
Japan 1 <1%
Croatia 1 <1%
Czechia 1 <1%
Canada 1 <1%
Unknown 110 95%

Demographic breakdown

Readers by professional status Count As %
Other 19 16%
Student > Postgraduate 18 16%
Researcher 17 15%
Student > Ph. D. Student 14 12%
Student > Master 14 12%
Other 34 29%
Readers by discipline Count As %
Medicine and Dentistry 69 59%
Unspecified 14 12%
Agricultural and Biological Sciences 11 9%
Biochemistry, Genetics and Molecular Biology 11 9%
Neuroscience 4 3%
Other 7 6%

Attention Score in Context

This research output has an Altmetric Attention Score of 2. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 06 January 2014.
All research outputs
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Outputs from Acta Neuropathologica
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Outputs of similar age
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Outputs of similar age from Acta Neuropathologica
of 27 outputs
Altmetric has tracked 12,884,100 research outputs across all sources so far. This one is in the 42nd percentile – i.e., 42% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,620 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 10.9. This one is in the 18th percentile – i.e., 18% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 249,172 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 55% of its contemporaries.
We're also able to compare this research output to 27 others from the same source and published within six weeks on either side of this one. This one is in the 25th percentile – i.e., 25% of its contemporaries scored the same or lower than it.